THE DOCTOR'S WORLD

THE DOCTOR'S WORLD; Rheumatic Fever Cases Posing Many Puzzles As Comeback Is Feared

By LAWRENCE K. ALTMAN, M.D.

Published: March 22, 1988

ACUTE rheumatic fever is a disease that most Americans have forgotten because it virtually disappeared from the United States a generation ago. Now, health officials worry that the disease, which is triggered by streptococcal sore throats, may be making a comeback. And its scattered reappearance is as mysterious as its disappearance.

In the last year, reports of outbreaks have come from Salt Lake City and the surrounding intermountain area; Columbus and Akron, Ohio, and Pittsburgh. The latest report is of 10 cases at a Navy training center in San Diego, the first there in more than 20 years.

So far, most of the outbreaks have involved small numbers of cases and have occurred in a spotty fashion. No one knows whether they are a mere blip in a declining incidence, or whether they herald a resurgence of a disease that can cripple, cause severe heart damage and kill.

What puzzles experts like Dr. Alan L. Bisno of the University of Miami and the Miami Veterans Administration Medical Center is why rheumatic fever is now striking primarily white middle-class children; a few decades ago most victims were poor.

Another puzzle is why almost all attacks follow sore throats caused by streptococcal bacteria, but only a few ''strep'' throats lead to rheumatic fever. The particular subgroup of strep bacteria cited in rheumatic fever cases is called group A. Rheumatic fever usually occurs in childhood but can cause long-term damage. In 1985, about 6,600 Americans died of heart disease resulting from bouts with the fever decades ago. There can be repeated attacks of rheumatic fever after additional attacks of strep throat; once the damage has occurred, it can be chronic and progressive.

Preventing the complication of rheumatic fever is why pediatricians impress parents with the importance of giving children a full course of antibiotic for a strep infection, even after it seems to have gone away.

A new concern is that rheumatic fever can be difficult to diagnose and that most younger American doctors have never seen a case. Some hospitals have begun holding conferences to reacquaint doctors with the strange way rheumatic fever acts.

The acute attacks begin one to four weeks after a strep throat. Doctors know that the streptococci do not migrate in the body to cause the attack of rheumatic fever, but they do not know how the bacteria set off the inflammatory reaction that occurs.

Because no laboratory tests are specific for rheumatic fever, doctors rely on a medical history, a physical examination and a classification scheme to diagnose it.

According to the scheme, known as the Jones criteria, there are five major and three minor indications. The major ones are: inflammation of the heart; arthritis; a syndrome involving involuntary jerky movements of the body, halting and slurred speech and facial grimaces, known as chorea, that disappear in a few weeks with no permanent damage; a rash that does not itch, and bumps under the skin.

The minor indications are fever, joint pains and a history of previous attacks of rheumatic fever. The presence of two major, or one major and two minor manifestations signals a high likelihood of rheumatic fever.

In a typical attack, rheumatic fever causes inflammation and swelling of the joints that is intensely painful for a few days; the pain abates over the next week as the swelling subsides. The usual targets are joints in the legs, particularly the knees, but the swelling and other symptoms often migrate from joint to joint.

Rheumatic fever does not permanently damage joints, but it can cause immediate fatal damage to the heart muscle, or it can produce long-term lethal effects by scarring valves. Damaged valves either restrict the blood flow or create severe leaks that strain the heart. When the scarring interferes with the opening and closing of the valves, it causes murmurs audible through a stethoscope. The scarred valves may require surgical repair or replacement.

Rheumatic fever was one of the most common diseases in America through the 1940's. In World War II, the rate reached 388 per 100,000 in some Army camps. The disease's decline began thereafter. By the mid-1960's, it affected about only 30 per 100,000 school children in mid-sized American cities. In surveys in the last few years, the rates were about 1 per 100,000. A similar decline occurred in Canada and Europe.

Over recent years, rheumatic fever sanitariums have closed for lack of patients. Registries established to track the disease have been abandoned, and many states have dropped rheumatic fever from their list of diseases that must be reported.

Yet throughout this period, rheumatic fever has remained one of the most common ailments in India, the Middle East and Africa, where in places it is the leading cause of death from heart disease.

What makes a disease like rheumatic fever show up again in developed countries? Theories abound, ranging from changes in virulence of streptococci to shifts in living standards, but no theory has been proved.

A common theory is that particular strains of streptococci cause rheumatic fever. But why such strains come and go is not known.

Epidemiologists have shown that household crowding increases the chances of spreading infection. Many have theorized that improvements in living standards led to the decline of rheumatic fever. But while crowded living conditions may still be a factor in some cases, Dr. Bisno, the Miami expert, said he could not point to any major change in housing conditions in recent years to explain the incidence of the disease.

The decline of rheumatic fever began before the introduction of penicillin, which led many experts to say that antibiotics were not the chief cause of its disappearance. Antibiotics have a limited role in preventing first attacks because many occur after strep throats that did not cause symptoms or were so mild that patients did not consult a physician. However, penicillin has been effective in preventing recurrent attacks of rheumatic fever.

Dr. Bisno said there was no convincing evidence of a decline in the incidence of streptococcal sore throats to parallel the decline in rheumatic fever. That has led some to suggest that a variety of viruses may act in concert to cause rheumatic fever.

Although doctors have suspected since 1889 that genetic factors might play a role in susceptibility to rheumatic fever, they have been unable to identify specific ones. Recently a research team headed by Dr. John B. Zabriskie of Rockefeller University identified two monoclonal antibodies that detected 92 percent of all rheumatic fever patients in a small group.

If the test proves accurate in larger groups, doctors may be able to prevent many cases of rheumatic fever by identifying individuals most susceptible to it and giving intene treatment to their strep throats.